This autosomal dominantly inherited forms of neurofibromatosis (NF) usually occurs without the visible stigmata of peripheral NF and is characterized by bilateral acoustic tumors. Complete families are being studied, including children, in order to provide earlier and more accurate diagnoses of tumors. Acoustic reflex studies and auditory brain stem response measures seem to be notably sensitive to VIII nerve neuroma. Clinical data suggest that either of these methods is likely to identify a small tumor when traditional CAT scanning measures may not. Appropriate and timely medical and audiological management plus counseling are considered for each patient individually.